alveolar proteinosis
Introduction
Introduction to alveolar proteinosis Alveolar proteinosis (PAP), also known as Rosen-Castle-man-Liebow syndrome. The alveolar and bronchial lumens are filled with PAS staining positive, and the phospholipid-rich protein substance from the lung is characterized by the fact that it occurs in young and middle-aged people, and the incidence of males is about three times that of females. basic knowledge The proportion of illness: 0.002% Susceptible people: no special people Mode of infection: non-infectious Complications: respiratory failure multiple lung infections
Cause
Causes of alveolar proteinosis
Cause:
The cause is unknown, and it is speculated that it is related to several factors: such as a large amount of dust inhalation (aluminum, silica, etc.), decreased immune function (especially infants and young children), genetic factors, alcoholism, microbial infections, etc., and sometimes it is difficult to confirm infection. Is the primary pathogenic factor or secondary to alveolar proteinosis, such as cytomegalovirus, Pneumocystis carinii, histoplasmosis infection, etc., found high alveolar protein deposition.
Although the triggering factor is still unclear, it basically agrees that the pathogenesis is caused by lipid metabolism disorder, that is, the metabolism of alveolar surfactant is abnormal due to the action of external factors in the body. So far, more alveolar macrophages have been studied. Cell viability, animal experiments show that the activity of macrophages after phagocytosis of dust is significantly reduced, and the macrophage particles in the patient's lavage fluid can reduce the normal cell viability, and the alveolar macrophage viability after bronchoalveolar lavage treatment. It can be raised, and the study found no increase in type II cell-producing protein and no abnormality in systemic lipid metabolism. Therefore, it is generally believed that this disease is associated with decreased clearance ability.
Prevention
Alveolar protein deposition prevention
1, to avoid infection with mycobacterial disease, Karst lung cyst pneumonia, cytomegalovirus and so on.
2, pay attention to exercise, improve immunity.
Complication
Alveolar proteinosis complications Complications respiratory failure multiple lung infections
Can be complicated by pulmonary infection and respiratory failure.
Symptom
Alveolar proteinosis symptoms common symptoms, fatigue, chest pain, purpura, difficulty breathing
The clinical manifestations of PAP are mostly non-specific, mostly manifested as progressive dyspnea, cough, cough and phlegm, secondary infection may be accompanied by fever and cough and purulent sputum, in addition to significant weakness, weight loss, loss of appetite, etc. Systemic symptoms, late manifestations of obvious shortness of breath and respiratory failure, signs are usually not obvious, occasionally smear and wet rales and sputum pronunciation, late can appear purpura and clubbing.
Examine
Examination of alveolar proteinosis
Chest X-ray can be expressed as diffuse nodules, patchy shadows or large patches.
Fiberoptic bronchoscopy biopsy, pathological examination, showed that the alveolar cavity was filled with PAS-positive coarse granular material, and alveolar lavage fluid showed a large number of amorphous fragments, often associated with PAS-positive macrophages.
Diagnosis
Diagnosis and identification of alveolar proteinosis
Diagnosis requires synergistic clinical manifestations, radiological signs and histopathology and / or alveolar lavage fluid cytology can be diagnosed, the diagnosis is based on: 1 clinical manifestations of activity after shortness of breath, occasional cough, progressive progressive symptoms, no infection And the basis of blood diseases; 2 chest X-ray can be expressed as diffuse nodules, patchy shadow or large solid shadow, HRCT visible map or paving stone characteristic changes; 3 fiberoptic bronchoscopy biopsy pathology The examination showed that the alveolar cavity was filled with PAS-positive coarse granular material, and a large number of amorphous fragments were observed in the alveolar lavage fluid, often accompanied by PAS-positive macrophages.
Pulmonary alveolar proteinosis is non-specific and should be differentiated from: 1 idiopathic pulmonary interstitial fibrosis; 2 alveolar carcinoma; 3 miliary tuberculosis; 4 lung parenchymal diseases such as viral pneumonia, mycoplasma pneumonia and chlamydia pneumonia.
